catecholaminergic polymorphic ventricular tachycardia

Noun

• an autosomal dominant inherited heart disorder caused by a mutation in voltage gated ion channels and resulting in arrhythmias. CPVT may cause exercise-induced ventricular arrhythmias and/or syncope occurring during physical activity or acute emotion, but demonstrates no structural problems of the heart. Ventricular tachycardia may self-terminate or degenerate into ventricular fibrillation, causing sudden death without immediate cardiopulmonary resuscitation. The majority of events occur during childhood and more than 60% of affected individuals will have a first episode of syncope or cardiac arrest by age 20.